Analysis Tools
behavior/neurological
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• mice exhibit hyperactive behavior
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hearing/vestibular/ear
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• mislocalization of the kinocilia is seen in inner hair cells (IHCs) and outer hair cells (OHCs) at prevalence of 45.5 and 50.9%, respectively
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• degenerated and decreased bundles are seen in most stereocilia
• stereocilia show several severely defective phenotypes in adults, such as loss of tapers, bulged bases, bulbous tips and gigantic bundles, all indications of stereociliary fusion
• stereocilia show abnormal formation, with misorientation in several hair cells and the bundling of adjacent stereocilia in the short population
• mice show abnormal elongation of the rootlets (distinct F-actin structures of the stereociliary base), with a more severe rootlet phenotype in the IHCs than the OHCs
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• stereocilia in in the inner hair cell exhibit fused giant stereocilia, bulged base, loss of tapered region and bulbous tip
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• mice show rapidly progressive stereociliary fusion of the cochlear hair cells during early postnatal stages such that stereocilia are completely fused by P1
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• orientations of the stereocilia bundles in utricle hair cells are irregular and the line of polarity reversal is difficult to identify at P7
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• mice show rapidly progressive stereociliary fusion of the cochlear hair cells during early postnatal stages such that stereocilia are completely fused by P14
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• decreased numbers of bundles are seen in most stereocilia
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• mice show raised and bulged cuticular plate membranes at the bases of stereociila in cochlear hair cells resulting in incorporation of the bundles into the sheaths of the cuticular plates
• the raised cuticular plate membranes in the IHCs occur earlier than those in the OHCs and are more severe
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• stereociliary degeneration of vestibular hair cells
• stereocilia show bulged basal region and a loss of tapered region
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• mice have no discernable ABR wave forms at any of the amplitudes tested
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• profound hearing loss
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nervous system
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• mislocalization of the kinocilia is seen in inner hair cells (IHCs) and outer hair cells (OHCs) at prevalence of 45.5 and 50.9%, respectively
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• degenerated and decreased bundles are seen in most stereocilia
• stereocilia show several severely defective phenotypes in adults, such as loss of tapers, bulged bases, bulbous tips and gigantic bundles, all indications of stereociliary fusion
• stereocilia show abnormal formation, with misorientation in several hair cells and the bundling of adjacent stereocilia in the short population
• mice show abnormal elongation of the rootlets (distinct F-actin structures of the stereociliary base), with a more severe rootlet phenotype in the IHCs than the OHCs
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• stereocilia in in the inner hair cell exhibit fused giant stereocilia, bulged base, loss of tapered region and bulbous tip
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• mice show rapidly progressive stereociliary fusion of the cochlear hair cells during early postnatal stages such that stereocilia are completely fused by P1
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• orientations of the stereocilia bundles in utricle hair cells are irregular and the line of polarity reversal is difficult to identify at P7
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• mice show rapidly progressive stereociliary fusion of the cochlear hair cells during early postnatal stages such that stereocilia are completely fused by P14
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• decreased numbers of bundles are seen in most stereocilia
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• stereociliary degeneration of vestibular hair cells
• stereocilia show bulged basal region and a loss of tapered region
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cellular
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• mislocalization of the kinocilia is seen in inner hair cells (IHCs) and outer hair cells (OHCs) at prevalence of 45.5 and 50.9%, respectively
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